Deck 1 Flashcards
What is often seen with respect to reticulocytes in DHTR in Sickle Cell Disease?
Reticulocytopenia
What are some common RBC antigens that lead to DHTR?
Kidd (Jk) and Rh
What is the goal HbF in someone started on hydroxyurea for SCD?
> 20%
Why are Sickle Cell patients immunocompromised?
Compromised function of the reticuloendothelial system and spleen + autoinfarction of the spleen
What may be used in severe cases of DHTR/Hyperhemolysis in Sickle Cell?
Steroids or IVIG
Though Staph is the MC cause of osteomyelitis, what proportion of osteomyelitis does it account for in Sickle Cell patients?
About 25%—bc mostly Salmonella
What is the most common anti-RBC antibody after ABO and Rh?
Anti-K (Kell)
What antigen on RBCs serves as the receptor for Plasmodium vivax and Plasmodium knowlesi?
Duffy antigen
When doing an exchange transfusion, the recommendation is to keep the Hgb no higher than ____. What is the HbS goal?
No higher than 10 g/dL; <30%
What is the idea behind IVIG (400 mg/kg) x5 days and/or steroids in patients with hyperhemolysis?
IVIG blocks adhesion of sickle cells to MO; steroids suppress MO
What is the normal methemoglobin level in a person with hemoglobin M disease?
15-30%
How would a reticulocyte count help in a person with hemolysis due to B12 deficiency?
It would be low as opposed to elevated in many causes of hemolysis
May also be low in Aplastic Anemia
What blood group antigen does the Parvovirus bind to on RBC precursors when it causes PRCA?
P antigen
What pregnancy category is iron dextran?
C
What iron formulation has a category B designation to administer to pregnant patients?
Iron Sucrose
In patients who develop ACS with SCD, what medication should be given to prevent future episodes?
Hydroxyurea (if not already on)
After a stroke in SCD, what percent of patients will recur without chronic transfusion? What about those getting chronic transfusion?
60% if do nothing; 20% if chronic transfuse to <30% Hgb S; 40% absolute risk reduction
What gene is mutated in Schwachman Diamond?
SBDS—increased cardiotoxicity to cyclophosphamide containing conditioning regimens
Prior to universal vaccination for S pneumoniae how much more likely were children with SCD to get it?
400x
What kind of gel is needed to differentiate HbA2 from HbC?
Citric Agar (they migrate together on normal agar)
HbE migrates with HbA2 on both
What is the normal inheritance pattern for pyruvate kinase deficiency? Gene?
Autosomal Recessive; PKLR
See reticulocytosis >50% after splenectomy
What drug is FDA approved for pyruvate kinase deficiency?
Mitapivat
What happens when a person with SCD and splenic sequestration gets a prbc transfusion? What should be done down the line?
It will regress—after acute episode, need to do splenectomy
In what year was hydrea approved by FDA for Sickle Cell?
1998